Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
Case Report
2001:67:1;46-47
PMID: 17664705

Primary cutaneous amyloidosis involving the external ears along with the classical sites

Binod K Khaitan, Apra Sood, Kaushal K Verma, M Ramam, Manoj K Singh
 Department of Dermatology and Venereology, and Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India

Correspondence Address:
Binod K Khaitan
Department of Dermatology and Venereology, and Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029
India
How to cite this article:
Khaitan BK, Sood A, Verma KK, Ramam M, Singh MK. Primary cutaneous amyloidosis involving the external ears along with the classical sites. Indian J Dermatol Venereol Leprol 2001;67:46-47
Copyright: (C)2001 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

A 26- year- old woman had multiple itchy persistent gradually progressive papular lesions on the forearms and shins for 10 and 4 years respectively. She also noticed similar lesions on both the ears for 4 years. There were no systemic symptoms. Cutaneous examination revealed multiple 2-3 mm discrete firm hyperpigmented papules on the extensors of forearms, shins and earlobes. Skin biopsy from all sites demonstrated deposits of amyloid in the papillary dermis. The patient was treated with cyclophosphamide 50 mg daily orally. There was more than 50% improvement in her lesions.
Keywords: Earlobe, Lichen amyoidosis, Cyclophosphamide

Introduction

Primary cutaneous amyloidosis manifests in various clinical forms. Macular, papular / lichenoid or a combination of the two are the common pre-sentation. Other rare forms are nodular or tumefactive, poikiloderma- like, bullous and vitiligi-nous.[1] Apart from the morphological variations, there are variations in the sites involved. The classical sites for lichen amyloidosis are shins, forearms and arms and for macular amyloidosis the upper back and upper arms.[1] We report lesions of amyloidosis in-volving both ears along with the lesions on the fore-arms and the shins in a young woman.

Case Report

A 26- year- old woman had multiple itchy persistent papules on the forearms that developed 10 years back. Four years later she noticed similar lesions on the shins. This was followed by appearance of mildly itchy papular lesions symmetrically on both the ear lobes. There were no systemic or con-stitutional, symptoms.

Exami-nation revealed multiple, monomorphic, discrete hyper-pigmented, firm, 2-3 mm p a pules localised on the extensor as-pects of both forearms [Figure - 1] and both legs (shins). Similar lesions were present inside the con-cha and the outer surface of upper part of pinna of both the ears. The other areas of the body, mucosae and scalp were normal. Examination of other sys-tems revealed no abnormalities. Investigations re-vealed haemoglobin -12 gm, total leucocyte count - 8000/mm3 with 64% neutrophils, 33% lymphocytes and 3% eosinophils. The ESR was 44 mm in first hour. Biochemical tests for renal and liver functions were within normal limits. Skin biopsy [Figure - 2] taken from the forearm skin as well as the ear lesions showed hyperkeratosis with mild acanthosis and amorphous hyalinized material in multiple adja-cent papillae. The stain with Congo red showed orange- red aggregated fibrillar material in the papillae un-der light microscopy and a bright green birefringence on polarising.

The patient was treated with cyclophosphamide 50 mg and cetirizine 10 mg daily orally along with topical clobetasol propionate 0.05% twice daily. Within 3 months, further progression of the disease was arrested along with reduction in the size of the lesions and significant relief in pruri-tus. Thereafter cetirizine was needed only occasion-ally. After 6 months of therapy there was more than 50% improvement in the extent of involvement and almost complete control of pruritus. No side- effects were observed with this treatment.

Discussion

The sites commonly involved in lichen amy-loidosis are pretibial areas, thighs, calves, ankles and dorsa of feet, while macular amyloidosis occurs mainly on the upper back (scapular and interscapular ar-eas), neck, clavicular areas and also the arms, fore-arms, thighs and shins.[1] In extensive cases, involve-ment of the face, chest, abdomen and anosacral region has also been recorded. The involvement of auricular concha in primary cutaneous amyloidosis is very rare. Perhaps, the first case in English litera-ture was reported in 1983 by Sanchez, as collag-enous papules on the aural conchae which were ac-tually primary cutaneous amyloidosis. Dupre et al[3] reported another case of aural lesions in 1984 where a similar case by Mascaro in Spanish literature was also referred. Hickes et al,[4] reported four such cases in 1988. In all these cases the amyloidosis was confined to ears and the other sites were uninvolved. However, in our case there was involvement of the forearms and shins along with the ex-ternal ears. The lesions were in au-ricular concha as well as on outer surface of pinna. This rare presenta-tion suggests that pathogenetically the process may be same but the in-volvement of various sites in one in-dividual and the variations in the dis-tribution in different individuals may possibly be because of the triggers pertaining to the affected sites or some kind of constitutional predis-position for these sites.

References
1.
Wang WJ. Clinical features of cutaneous amyloidoses. Clin Dermatol 1990; 8:13-19.
[Google Scholar]
2.
Sanchez JL. Collagenous papules on the aural conchae. Am J Dermatopathol 1983;5:231-223.
[Google Scholar]
3.
Dupre A, Lassere J, Bonafe JL, et al. Papules collagenes de la Conque des Oreilles. Ann Dermatol Venereol 1984;111: 913-917.
[Google Scholar]
4.
Hicks BS, Weber PJ, Hashimoto K, et al. Primary cutaneous amyloidoses of the auricular concha. J Am Acad Dermatol 1998; 19:19-25.
[Google Scholar]
Show Sections